- Email: [email protected]
Vulvar Fibroepithelial Polyps in a Female Adolescent: A Case Report
Accepted Manuscript Vulvar Fibroepithelial Polyps in an Adolescent Female Jonathan Avila, MD, Kathleen Nicol, MD, Steven C. Matson, MD PII:
S1083-3188(17)30042-6
DOI:
10.1016/j.jpag.2017.04.004
Reference:
PEDADO 2122
To appear in:
Journal of Pediatric and Adolescent Gynecology
Received Date: 10 February 2017 Revised Date:
30 March 2017
Accepted Date: 24 April 2017
Please cite this article as: Avila J, Nicol K, Matson SC, Vulvar Fibroepithelial Polyps in an Adolescent Female, Journal of Pediatric and Adolescent Gynecology (2017), doi: 10.1016/j.jpag.2017.04.004. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Vulvar Fibroepithelial Polyps in an Adolescent Female
RI PT
A Case Report
Jonathan Avila MD
Nationwide Children’s Hospital Pediatrics Residency Program, Columbus, Ohio
SC
Kathleen Nicol MD
M AN U
Department of Pathology & Laboratory Medicine, Nationwide Children’s Hospital, Columbus, Ohio
Steven C. Matson, MD
Chief, Division of Adolescent Medicine Nationwide Children’s Hospital
Associate Professor of Pediatrics
The Ohio State University College of Medicine
TE D
700 Children’s Drive, G361 Timken Hall Columbus, OH 43205-2664 [email protected]
Fax: 614-355-3583
AC C
EP
Phone: 614-722-2493
1
ACCEPTED MANUSCRIPT
Abstract Background: Fibroepithelial polyps (FEPs) are benign tumors, of possibly hormone-
RI PT
dependent nature, found in the vulvovaginal region of women of reproductive age. Case: A 15-year-old adolescent girl, on hormonal contraceptive therapy, who
presented with multiple vulvar masses with histopathology consistent with FEP.
SC
Summary and Conclusion: The spectrum of the morphology of FEPs may make their diagnosis challenging. We describe a rare presentation of vulvar fibroepithelial polyps
M AN U
in an adolescent girl on hormonal contraceptive therapy.
AC C
EP
TE D
Key Words: vulva; genital mass; fibroepithelial polyp; adolescent; soft tissue tumor
2
ACCEPTED MANUSCRIPT
Introduction Norris and Taylor provided one of the earliest descriptions of distinctive, benign
RI PT
stromal polyps of the vagina in 1966.1 Fibroepithelial stromal polyp, is a benign polypoid growth of the vagina (most common), vulva or cervix that is strongly tied to hormonal stimulation. It occurs most often during pregnancy but may also be found in reproductive age women or postmenopausal women undergoing hormone replacement
SC
therapy.2 Their wide range of morphologic appearance may pose diagnostic
M AN U
difficulties. They are usually solitary lesions, but there may be multiple polyps at presentation, especially in pregnant patients. They are usually polypoid or pedunculated, but may exhibit multiple finger-like projection, thus mimicking a condyloma.2 The typical clinical presentation of this entity is the finding of one or more polyps that may be symptomatic by causing bleeding, discharge or discomfort
as 18.5 cm.3
TE D
depending on the size of the lesion, usually 1–5 cm in diameter, but may be as large
EP
The pathogenesis of FEPs is not well understood, but a hormonal association has been proposed. Evidence suggesting that hormonal influences play a role in their
AC C
formation includes: (1) the propensity of these lesions to occur during pregnancy and spontaneously regress following delivery; (2) the association with hormone therapy; and (3) the presence of estrogen and progesterone receptor positive FEP stromal cells.4 We report the case of an adolescent girl, on hormonal contraceptive therapy, who presented with a large, pedunculated vulvar mass amidst other smaller, polypoid vulvar lesions of histopathology consistent with a FEP. Consent to publish clinically related images was obtained from the patient. 3
ACCEPTED MANUSCRIPT
Case A 15-year-old nulligravid African American female presented to the Adolescent
RI PT
Medicine Clinic for evaluation of a right labial mass. During her well child visit at age 9 years and 11 months she was noted to have entered puberty with Tanner 2 breasts and Tanner 3 pubic hair. The patient first noticed several “bumps” on her labia at around age 10 to 11 years old. The bumps have progressively increased in size since
SC
onset. About 2 to 3 weeks prior to presentation, one of the growths ruptured
M AN U
spontaneously with bloody drainage; the lesion healed, but the mass remained. The masses have not bothered her, except for their appearance. They were neither painful nor tender; and did not cause any irritation or burning sensation. There was no associated vaginal bleeding, discharge or odor; no dysuria, dyspareunia, urinary
TE D
incontinence, or hematuria.
The patient had a history of regular menstrual cycles, with menarche at age 12 years. Her cycle length averaged 4 to 5 days, without dysmenorrhea. She was
EP
sexually active with one male partner at time of presentation, with coitarche at age 14 years. She reported using condoms consistently. At age 14 years and 3 months she
AC C
initiated 150 mg of depot medroxyprogesterone acetate (DMPA) IM injections every 12 weeks as a contraceptive method. She had received 6 injections prior to presenting with the mass. She had no history of sexually transmitted infections (STI) including HSV or genital warts. Her last STI screen, 5 months prior to presentation, was negative for Neisseria gonorrhoeae, Chlamydia trachomatis, or Trichomonas vaginalis. She had completed her HPV vaccine series 4 years prior to presentation. She was a nonsmoker, and denied any significant alcohol or drug use. Her medical 4
ACCEPTED MANUSCRIPT
and surgical history was otherwise noncontributory. Her family history was significant for systemic lupus erythematosus and uterine fibroids in second-degree relatives. Although these lesions must have grown while she was receiving DMPA, it was not
RI PT
clear if growth actually accelerated while receiving this contraceptive. Her physical exam was remarkable for a large, non-tender, skin-colored, ulcerating pedunculated mass of 3.8 cm (largest diameter) extending from the right labia majora; with
SC
smaller, polypoid, skin-colored papules in the adjacent region in the ipsilateral labium
at that visit was negative.
M AN U
(Figure 1). There was no inguinal lymphadenopathy. Urine pregnancy test obtained
The patient was referred to pediatric gynecology, who performed surgical excision of her right vulvar masses under general anesthesia (Figure 2). Microscopic evaluation of the excised masses confirmed their histopathology to be consistent with
TE D
fibroepithelial polyps (Figure 3).
After resection of the FEPs she has continued on DMPA and has received 8 more
EP
injections at this time (age 17 years and 5 months), with no recurrence of her lesions. Although some FEPs seem to be hormone related it was felt that prevention of
AC C
unwanted pregnancy was more important than possible recurrence of the masses.
Summary and Conclusion In this case report we describe a rare presentation of vulvar fibroepithelial polyps in an adolescent girl on hormonal contraceptive therapy. Further unusual
5
ACCEPTED MANUSCRIPT
features included the uncommon location on the vulva, and presence of multiple smaller lesions with the more prominent larger mass.
RI PT
Since FEPs appear in the medical literature as sporadic case reports, their incidence in the population, and their ethnic and genetic predisposition is uncertain. Case studies describe these lesions as uncommon,5 whereas others suggest that these lesions may actually be more common than typically thought.6 The fact that FEPs
SC
refer to a spectrum of benign lesions that may arise from either the vulvovaginal skin
M AN U
or mucosa make epidemiologic studies even more difficult.
FEPs are usually asymptomatic, and therefore found incidentally during a routine gynecologic exam, or reported by the patient due to concerns for their appearance. When symptomatic, bleeding is a very common presentation,4 as
TE D
reported, for instance, by the patient in this case report. When ulceration occurs, inflammation secondary to infection may occur,5 such as found in our patient. That hormonal stimulation may lead to these lesions is supported by the
EP
discovery of FEPs in patients who are pregnant, with regression of these lesions after the puerperium period,6 as well as in patients on hormone therapy, or therapy with
AC C
hormone receptor modulators. Immunohistochemical staining of FEPs confirm expression of receptors for estrogen and progesterone. This association suggests a hormone-dependent nature, but no studies in adolescent patients with FEPs has been done to this date to evaluate this possibility. No malignant transformation has been observed in these lesions,6 therefore treatment may not be necessary for smaller lesions. Treatment of larger FEPs,
6
ACCEPTED MANUSCRIPT
however, may help relieve the anxiety patients may develop surrounding these genital lesions. A simple surgical excision is typically curative, as well as helpful for histological confirmation of their benign nature, especially with large lesions, which
RI PT
may present similar to aggressive angiomyxoma or botryoid variant of embryonal
rhabdomyosarcoma, especially in pediatric patients.6 Reoccurrence after excision may happen.7
SC
Fibroepithelial polyps of the vulva are common in women of reproductive age.
M AN U
Although individual reports of these lesions in adolescents and infants exist, most lesions are found in older women. These lesions are usually small, and histologically benign. Larger lesions are much less common and likely arise from proliferation of mesenchymal cells within the hormonally sensitive subepithelial stromal layer of the lower genital tract. Expert pathological interpretation may be necessary to exclude
AC C
EP
TE D
lesions such as aggressive angiomyxoma, angiomyofibroblastoma and sarcoma.2,8
7
ACCEPTED MANUSCRIPT
Appendix Exophytic large mass and smaller flat masses on right labia of patient
Figure 2.
Post-operative photo after masses excised
Figure 3.
Low power microscopic and high power pathology of mass showing, “A
RI PT
Figure 1.
polypoid portion of soft tissue covered by hyperplastic keratinized
SC
squamous epithelium and having a core composed of loose collagen with
AC C
EP
TE D
M AN U
increased blood vessels.”
8
ACCEPTED MANUSCRIPT
References
1. Norris HJ, Taylor HB: Polyps of the vagina. A benign lesion resembling sarcoma
RI PT
botryoides. Cancer 1966; 19:227–232
2. Nucci MR, Young RH, Fletcher CD: Cellular pseudosarcomatous fibroepithelial
SC
stromal polyps of the lower female genital tract: an underrecognized lesion often
M AN U
misdiagnosed as sarcoma. Am J Surg Pathol 2000; 24:231
3. Schoolmeester JK, Fritchie KJ: Genital soft tissue tumors. J Cutan Pathol 2015; 42:441–451
TE D
4. Nucci MR, Fletcher CDM: Vulvovaginal soft tissue tumours: update and review. Histopathology 2000; 36:97–108
EP
5. Navada MH, Bhat PRB, Rao SV, et al: Large fibroepithelial polyp of vulva. Case Rep
AC C
in Dermatol Med 2011; Article ID 273181, 2 pages
6. McCluggage W: A review and update of morphologically bland vulvovaginal mesenchymal lesions. Int J Gynecol Pathol 2005; 24:26-38
7. Nielsen GP, Young RH: Mesenchymal tumors and tumor-like lesions of the female genital tract: a selective review with emphasis on recently described entities. Int J Gynecol Pathol 2001; 20:105-127 9
ACCEPTED MANUSCRIPT
8. Laskin W, Fetsch J, Tavassoli F. Superficial cervicovaginal myofibroblastoma:
RI PT
fourteen cases of a distinctive mesenchymal tumour arising from the specialized
AC C
EP
TE D
M AN U
SC
subepithelial stroma of the lower female genital tract. Hum Pathol 2001;32:715–25
10
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
Figure 1
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
Figure 2
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
Figure 3 Low (A) and high (B) power miscropic pathology of mass showing “A polypoid portion of soft tissue covered by hyperplastic keratinized squamous epithelium and having a core composed of loose collagen with increased blood vessels”.
S1083-3188(17)30042-6
DOI:
10.1016/j.jpag.2017.04.004
Reference:
PEDADO 2122
To appear in:
Journal of Pediatric and Adolescent Gynecology
Received Date: 10 February 2017 Revised Date:
30 March 2017
Accepted Date: 24 April 2017
Please cite this article as: Avila J, Nicol K, Matson SC, Vulvar Fibroepithelial Polyps in an Adolescent Female, Journal of Pediatric and Adolescent Gynecology (2017), doi: 10.1016/j.jpag.2017.04.004. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Vulvar Fibroepithelial Polyps in an Adolescent Female
RI PT
A Case Report
Jonathan Avila MD
Nationwide Children’s Hospital Pediatrics Residency Program, Columbus, Ohio
SC
Kathleen Nicol MD
M AN U
Department of Pathology & Laboratory Medicine, Nationwide Children’s Hospital, Columbus, Ohio
Steven C. Matson, MD
Chief, Division of Adolescent Medicine Nationwide Children’s Hospital
Associate Professor of Pediatrics
The Ohio State University College of Medicine
TE D
700 Children’s Drive, G361 Timken Hall Columbus, OH 43205-2664 [email protected]
Fax: 614-355-3583
AC C
EP
Phone: 614-722-2493
1
ACCEPTED MANUSCRIPT
Abstract Background: Fibroepithelial polyps (FEPs) are benign tumors, of possibly hormone-
RI PT
dependent nature, found in the vulvovaginal region of women of reproductive age. Case: A 15-year-old adolescent girl, on hormonal contraceptive therapy, who
presented with multiple vulvar masses with histopathology consistent with FEP.
SC
Summary and Conclusion: The spectrum of the morphology of FEPs may make their diagnosis challenging. We describe a rare presentation of vulvar fibroepithelial polyps
M AN U
in an adolescent girl on hormonal contraceptive therapy.
AC C
EP
TE D
Key Words: vulva; genital mass; fibroepithelial polyp; adolescent; soft tissue tumor
2
ACCEPTED MANUSCRIPT
Introduction Norris and Taylor provided one of the earliest descriptions of distinctive, benign
RI PT
stromal polyps of the vagina in 1966.1 Fibroepithelial stromal polyp, is a benign polypoid growth of the vagina (most common), vulva or cervix that is strongly tied to hormonal stimulation. It occurs most often during pregnancy but may also be found in reproductive age women or postmenopausal women undergoing hormone replacement
SC
therapy.2 Their wide range of morphologic appearance may pose diagnostic
M AN U
difficulties. They are usually solitary lesions, but there may be multiple polyps at presentation, especially in pregnant patients. They are usually polypoid or pedunculated, but may exhibit multiple finger-like projection, thus mimicking a condyloma.2 The typical clinical presentation of this entity is the finding of one or more polyps that may be symptomatic by causing bleeding, discharge or discomfort
as 18.5 cm.3
TE D
depending on the size of the lesion, usually 1–5 cm in diameter, but may be as large
EP
The pathogenesis of FEPs is not well understood, but a hormonal association has been proposed. Evidence suggesting that hormonal influences play a role in their
AC C
formation includes: (1) the propensity of these lesions to occur during pregnancy and spontaneously regress following delivery; (2) the association with hormone therapy; and (3) the presence of estrogen and progesterone receptor positive FEP stromal cells.4 We report the case of an adolescent girl, on hormonal contraceptive therapy, who presented with a large, pedunculated vulvar mass amidst other smaller, polypoid vulvar lesions of histopathology consistent with a FEP. Consent to publish clinically related images was obtained from the patient. 3
ACCEPTED MANUSCRIPT
Case A 15-year-old nulligravid African American female presented to the Adolescent
RI PT
Medicine Clinic for evaluation of a right labial mass. During her well child visit at age 9 years and 11 months she was noted to have entered puberty with Tanner 2 breasts and Tanner 3 pubic hair. The patient first noticed several “bumps” on her labia at around age 10 to 11 years old. The bumps have progressively increased in size since
SC
onset. About 2 to 3 weeks prior to presentation, one of the growths ruptured
M AN U
spontaneously with bloody drainage; the lesion healed, but the mass remained. The masses have not bothered her, except for their appearance. They were neither painful nor tender; and did not cause any irritation or burning sensation. There was no associated vaginal bleeding, discharge or odor; no dysuria, dyspareunia, urinary
TE D
incontinence, or hematuria.
The patient had a history of regular menstrual cycles, with menarche at age 12 years. Her cycle length averaged 4 to 5 days, without dysmenorrhea. She was
EP
sexually active with one male partner at time of presentation, with coitarche at age 14 years. She reported using condoms consistently. At age 14 years and 3 months she
AC C
initiated 150 mg of depot medroxyprogesterone acetate (DMPA) IM injections every 12 weeks as a contraceptive method. She had received 6 injections prior to presenting with the mass. She had no history of sexually transmitted infections (STI) including HSV or genital warts. Her last STI screen, 5 months prior to presentation, was negative for Neisseria gonorrhoeae, Chlamydia trachomatis, or Trichomonas vaginalis. She had completed her HPV vaccine series 4 years prior to presentation. She was a nonsmoker, and denied any significant alcohol or drug use. Her medical 4
ACCEPTED MANUSCRIPT
and surgical history was otherwise noncontributory. Her family history was significant for systemic lupus erythematosus and uterine fibroids in second-degree relatives. Although these lesions must have grown while she was receiving DMPA, it was not
RI PT
clear if growth actually accelerated while receiving this contraceptive. Her physical exam was remarkable for a large, non-tender, skin-colored, ulcerating pedunculated mass of 3.8 cm (largest diameter) extending from the right labia majora; with
SC
smaller, polypoid, skin-colored papules in the adjacent region in the ipsilateral labium
at that visit was negative.
M AN U
(Figure 1). There was no inguinal lymphadenopathy. Urine pregnancy test obtained
The patient was referred to pediatric gynecology, who performed surgical excision of her right vulvar masses under general anesthesia (Figure 2). Microscopic evaluation of the excised masses confirmed their histopathology to be consistent with
TE D
fibroepithelial polyps (Figure 3).
After resection of the FEPs she has continued on DMPA and has received 8 more
EP
injections at this time (age 17 years and 5 months), with no recurrence of her lesions. Although some FEPs seem to be hormone related it was felt that prevention of
AC C
unwanted pregnancy was more important than possible recurrence of the masses.
Summary and Conclusion In this case report we describe a rare presentation of vulvar fibroepithelial polyps in an adolescent girl on hormonal contraceptive therapy. Further unusual
5
ACCEPTED MANUSCRIPT
features included the uncommon location on the vulva, and presence of multiple smaller lesions with the more prominent larger mass.
RI PT
Since FEPs appear in the medical literature as sporadic case reports, their incidence in the population, and their ethnic and genetic predisposition is uncertain. Case studies describe these lesions as uncommon,5 whereas others suggest that these lesions may actually be more common than typically thought.6 The fact that FEPs
SC
refer to a spectrum of benign lesions that may arise from either the vulvovaginal skin
M AN U
or mucosa make epidemiologic studies even more difficult.
FEPs are usually asymptomatic, and therefore found incidentally during a routine gynecologic exam, or reported by the patient due to concerns for their appearance. When symptomatic, bleeding is a very common presentation,4 as
TE D
reported, for instance, by the patient in this case report. When ulceration occurs, inflammation secondary to infection may occur,5 such as found in our patient. That hormonal stimulation may lead to these lesions is supported by the
EP
discovery of FEPs in patients who are pregnant, with regression of these lesions after the puerperium period,6 as well as in patients on hormone therapy, or therapy with
AC C
hormone receptor modulators. Immunohistochemical staining of FEPs confirm expression of receptors for estrogen and progesterone. This association suggests a hormone-dependent nature, but no studies in adolescent patients with FEPs has been done to this date to evaluate this possibility. No malignant transformation has been observed in these lesions,6 therefore treatment may not be necessary for smaller lesions. Treatment of larger FEPs,
6
ACCEPTED MANUSCRIPT
however, may help relieve the anxiety patients may develop surrounding these genital lesions. A simple surgical excision is typically curative, as well as helpful for histological confirmation of their benign nature, especially with large lesions, which
RI PT
may present similar to aggressive angiomyxoma or botryoid variant of embryonal
rhabdomyosarcoma, especially in pediatric patients.6 Reoccurrence after excision may happen.7
SC
Fibroepithelial polyps of the vulva are common in women of reproductive age.
M AN U
Although individual reports of these lesions in adolescents and infants exist, most lesions are found in older women. These lesions are usually small, and histologically benign. Larger lesions are much less common and likely arise from proliferation of mesenchymal cells within the hormonally sensitive subepithelial stromal layer of the lower genital tract. Expert pathological interpretation may be necessary to exclude
AC C
EP
TE D
lesions such as aggressive angiomyxoma, angiomyofibroblastoma and sarcoma.2,8
7
ACCEPTED MANUSCRIPT
Appendix Exophytic large mass and smaller flat masses on right labia of patient
Figure 2.
Post-operative photo after masses excised
Figure 3.
Low power microscopic and high power pathology of mass showing, “A
RI PT
Figure 1.
polypoid portion of soft tissue covered by hyperplastic keratinized
SC
squamous epithelium and having a core composed of loose collagen with
AC C
EP
TE D
M AN U
increased blood vessels.”
8
ACCEPTED MANUSCRIPT
References
1. Norris HJ, Taylor HB: Polyps of the vagina. A benign lesion resembling sarcoma
RI PT
botryoides. Cancer 1966; 19:227–232
2. Nucci MR, Young RH, Fletcher CD: Cellular pseudosarcomatous fibroepithelial
SC
stromal polyps of the lower female genital tract: an underrecognized lesion often
M AN U
misdiagnosed as sarcoma. Am J Surg Pathol 2000; 24:231
3. Schoolmeester JK, Fritchie KJ: Genital soft tissue tumors. J Cutan Pathol 2015; 42:441–451
TE D
4. Nucci MR, Fletcher CDM: Vulvovaginal soft tissue tumours: update and review. Histopathology 2000; 36:97–108
EP
5. Navada MH, Bhat PRB, Rao SV, et al: Large fibroepithelial polyp of vulva. Case Rep
AC C
in Dermatol Med 2011; Article ID 273181, 2 pages
6. McCluggage W: A review and update of morphologically bland vulvovaginal mesenchymal lesions. Int J Gynecol Pathol 2005; 24:26-38
7. Nielsen GP, Young RH: Mesenchymal tumors and tumor-like lesions of the female genital tract: a selective review with emphasis on recently described entities. Int J Gynecol Pathol 2001; 20:105-127 9
ACCEPTED MANUSCRIPT
8. Laskin W, Fetsch J, Tavassoli F. Superficial cervicovaginal myofibroblastoma:
RI PT
fourteen cases of a distinctive mesenchymal tumour arising from the specialized
AC C
EP
TE D
M AN U
SC
subepithelial stroma of the lower female genital tract. Hum Pathol 2001;32:715–25
10
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
Figure 1
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
Figure 2
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
Figure 3 Low (A) and high (B) power miscropic pathology of mass showing “A polypoid portion of soft tissue covered by hyperplastic keratinized squamous epithelium and having a core composed of loose collagen with increased blood vessels”.